Perioperative Care in Patients with Ehlers Danlos Syndromes

Abstract

Background: There is an increasing recognition of patients with Ehlers Danlos Syndromes. The laxity of the ligaments and the weakness of the connective tissue has resulted in increasing number of patients requiring surgical intervention. Ehlers Danlos Syndromes are not about hypermobile joints only, they are associated with multiple co-existing conditions such as Chiari malformation, Tethered Cord Syndrome, spinal instability, abdominal pain, Dysautonomia and Mast Cell Activation Syndrome. The combined incidence of Ehlers Danlos Syndromes is 1 in 5000 people. Most experts believe that the actual incidence is much higher. Many of these cases are under-diagnosed. Nevertheless, patients with Ehlers Danlos Syndromes, diagnosed or undiagnosed often require surgical intervention. This review article has been written to shed light on the need for special consideration during anesthesia. Objectives: Our objective was to conduct a review of anesthetic considerations in patients with Ehlers Danlos Syndromes. Study Design: We used a narrative review design. Methods: This review was done using searches of PubMed, MEDLINE/OVID, SCOPUS, and manual searches of the bibliographies of known primary and review articles from inception to 2019. Other data sources included hand searches of publications driven by manuscript authors. Search terms included concepts of “Ehlers Danlos Syndrome”, “EDS”, “pain”, “anesthesia”, “surgery” and combination of terms. Search method was not restricted to any one language. Results: Articles were screened by title, abstract, and full article review. They were then analyzed by specific clinical indications and appropriate data was presented based on critical analysis of those articles. Limitations: More studies about the effect of anesthetic techniques and Ehlers Danlos Syndromes are required. Conclusions: Patients with Ehlers Danlos Syndromes may present with an array of coexisting medical conditions such as Dysautonomia, Mast Cell Activation Syndrome, Chiari Malformation, Tethered Cord Syndrome, Craniocervical instability, Gastroparesis, altered sensitivity to local anesthetics. Anesthetic techniques need to be modified according to the presentation. Preoperatively, a note should be made of the joints that sublux most often, craniocervical instability. They are prone to temporomandibular joint subluxation, obstructive sleep apnea and tracheomalacia, spontaneous pneumothorax, POTS, MCAS, insensitivity to local anesthetics and coagulation disorders. Intraoperative considerations should include fiberoptic intubation, IV fluid loading for POTS, avoid drugs that release histamine, unpredictable response to opioids, precautions for gastroparesis. Patient should be positioned to avoid joint subluxation and over stretching nerves. Postoperative considerations include maintaining hemodynamic stability, avoid exacerbation of symptoms of MCAS, high suspicion of occult bleeding, high risk of post dural puncture headache in patients undergoing spinal procedure or neuroaxial block.